Investigation of cellular toxicity induced by LIN28 in amyotrophic lateral sclerosis

• Project/Area Number: 23791002
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Neurology
• Research Institution: Keio University
• Principal Investigator: NISHIMOTO Yoshinori 慶應義塾大学, 医学部, 特任助教 (30398622)
• Project Period (FY): 2011 – 2012
• Project Status: Completed (Fiscal Year 2012)
• Budget Amount: ¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000); Fiscal Year 2012: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000); Fiscal Year 2011: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
• Keywords: 筋萎縮性側索硬化症 (ALS) / TDP-43 / 胚性幹細胞 (ES 細胞) / 運動神経細胞死 / 筋萎縮性側索硬化症 / ALS / 胚性幹細胞 / ES細胞 / マグネトフェクション / RNA編集 / LIN28 / ADAR2 / 胚性幹細胞 (ES細胞) / 運動神経細胞

Research Abstract

We established the procedures of transfection into human motor neurons derived from embryonic stem (ES) cells, of which magnetofection was proved most useful. Taking advantage of this procedure, 26k-Da C terminal isoform and mutant forms of TDP-43, that i

Research Products

• [Journal Article] Cellular toxicity induced by the 26-kDa fragment and amyotrophic lateral sclerosis (ALS)-associated mutant forms of TDP-43 in human embryonic stem cell-derived motor neurons (2013)
  • Author(s): Yoshinori Nishimoto, Hirotaka J. Okano, Takao Imai, Aleksandra J. Poole, Norihiro Suzuki, Hans S. Keirstead, Hideyuki Okano
  • Journal Title: Neurology and Clinical Neuroscience Volume: 1(1) Pages: 24-31
  • Peer Reviewed
• [Journal Article] Cellular toxicity induced by the 26-kDa fragment and amyotrophic lateral sclerosis (ALS)-associated mutant forms of TDP-43 in human embryonic stem cell-derived motor neurons. (2013)
  • Author(s): Yoshinori Nishimoto
  • Journal Title: Neurology and Clinical Neuroscience Volume: 1 Pages: 24-31
  • Peer Reviewed
• [Journal Article] RNA editing of the Q/R site of GluA2 in cultured cell lines expressing different levels of the RNA editing enzyme ADAR2 (2012)
  • Author(s): Yamashita T, Tadami C, Nishimoto Y, Hideyama T, Kimura D, Suzuki T,Kwak S
  • Journal Title: Neurosci Res Volume: 73 Issue: 1 Pages: 42-48
  • DOI: 10.1016/j.neures.2012.02.002
  • Peer Reviewed
• [Journal Article] RNA-Binding Protein Musashi1 Modulates Glioma Cell Growth through the Post-Transcriptional Regulation of Notch and PI(3) Kinase/Akt Signaling Pathways (2012)
  • Author(s): Muto J, Imai T, Ogawa D, Nishimoto Y, Okada Y, Mabuchi Y, Kawase T, Iwanami A, Mischel PS, Saya H, Yoshida K, Matsuzaki Y, Okano H
  • Journal Title: PLoS One Volume: 7(3) Issue: 3 Pages: e33431-e33431
  • DOI: 10.1371/journal.pone.0033431
  • Peer Reviewed
• [Journal Article] 一過性全健忘でのヒト海馬CA1領域の限局性病変は空間ナビゲーション学習を障害する (2011)
  • Author(s): 西本祥仁, 伊東大介
  • Journal Title: MEDICAL BRIEFS IN Brain & Nerve Volume: 18 Pages: 6-7
• [Journal Article] Ataxin-2グルタミン鎖伸長と神経変性疾患－ALS, PSP発症リスクとの相関性について (2011)
  • Author(s): 西本祥仁, 伊東大介
  • Journal Title: Cognition and Dementia Volume: 10 Pages: 58-59
• [Journal Article] microRNA-206によるALSモデルマウスの疾患進行抑制と神経筋接合部におけるシナプス再生の促進 (2011)
  • Author(s): 西本祥仁
  • Journal Title: MEDICAL BRIEFS IN Brain & Nerve Volume: 18 Pages: 8-9
• [Journal Article] A novel mutation in the HTRA1 gene causes CARASIL without alopecia. (2011)
  • Author(s): Nishimoto Y,
  • Journal Title: Neurology. Volume: 76 Issue: 15 Pages: 1353-1355
  • DOI: 10.1212/wnl.0b013e318215281d
  • Peer Reviewed
• [Journal Article] Headache associated with myasthenia gravis: the impact of mild ocular symptoms. (2011)
  • Author(s): Yoshinori Nishimoto, Shigeaki Suzuki, Kimiaki Utsugisawa, Yuriko Nagane, Mamoru Shibata, Toshihiko Shimizu, Norihiro Suzuki.
  • Journal Title: Autoimmune Diseases Volume: 2011 Pages: 840364-840364
  • DOI: 10.4061/2011/840364
  • Peer Reviewed
• [Journal Article] Slow-progressive ataxia with a methionine-to-arginine point mutation in codon 232 in the prion protein gene (PRNP). (2011)
  • Author(s): Yoshinori Nishimoto, Daisuke Ito, Shigeaki Suzuki, Toshihiko Shimizu, Tetsuyuki Kitamoto and Norihiro Suzuki.
  • Journal Title: Clinical Neurology and Neurosurgery Volume: 113 Issue: 8 Pages: 696-698
  • DOI: 10.1016/j.clineuro.2011.04.009
  • Peer Reviewed
• [Journal Article] Identification of a Novel Intronic Enhancer Responsible for the Transcriptional Regulation of Musashi1 in Neural Stem/Progenitor Cells (2011)
  • Author(s): Kawase S, Imai T, Miyauchi-Hara C, Yaguchi K, Nishimoto Y, Fukami SI, Matsuzaki Y, Miyawaki A, Itohara S, Okano H
  • Journal Title: Mol Brain Volume: 4(1) Issue: 1 Pages: 14-14
  • DOI: 10.1186/1756-6606-4-14
  • Peer Reviewed
• [Journal Article] Neurological picture. Neuroaxonal integrity evaluated by MR spectroscopy in a case of CARASIL. (2011)
  • Author(s): Nishimoto Y,
  • Journal Title: J Neurol Neurosurg Psychiatry. Volume: 82 Issue: 8 Pages: 860-861
  • DOI: 10.1136/jnnp.2010.240051
  • Peer Reviewed
• [Presentation] 孤発性ALS の運動神経細胞において特異的に出現する核内構造体の同定 (2013)
  • Author(s): 西本祥仁, 鈴木則宏, 岡野栄之
  • Organizer: 第54回日本神経学会学術大会
  • Place of Presentation: 東京国際フォーラム
  • Year and Date: 2013-05-31
• [Presentation] ヒト胚性幹細胞由来運動神経細胞での TDP-43 発現による細胞毒性の検討 (2012)
  • Author(s): 西本祥仁, 岡野ジェイムス洋尚, 今井貴雄, 鈴木則宏, 岡野栄之
  • Organizer: 第53回日本神経学会学術大会
  • Place of Presentation: 東京国際フォーラム
  • Year and Date: 2012-05-25
• [Presentation] 'Investigation into cellular toxicity induced by TDP-43 expression in human ES cell-derived motor neurons' poster presentation (2012)
  • Author(s): Yoshinori Nishimoto, Hirotaka James Okano, Takao Imai and Hideyuki Okano
  • Organizer: The 4th symposium on Brain and Mind Research in the Asia-Pacific (BMAP 2012)
  • Place of Presentation: Keio University, Mita, Tokyo
• [Presentation] Investigation into cellular toxicity induced by TDP-43 expression in human ES cell-derived motor neurons’ poster presentation (2012)
  • Author(s): Yoshinori Nishimoto
  • Organizer: The 4th symposium on Brain and Mind Research in the Asia-Pacific (BMAP 2012)
  • Place of Presentation: Keio University, Mita, Tokyo
• [Presentation] 禿頭を欠くCARASILにおいて認められた新規HTRA1遺伝子変異に関する分子生物学的考察 (2011)
  • Author(s): 西本祥仁、柴田護、二本松萌、野崎洋明、志賀篤、白田明子、山根清美、小堺有史、高橋一司、西澤正豊、小野寺理、鈴木則宏
  • Organizer: 第52回 日本神経学会学術大会
  • Place of Presentation: 名古屋国際会議場 (愛知県)
• [Presentation] CARASILのMRI画像 (2011)
  • Author(s): 野崎洋明、福武敏夫、下江豊、平山幹生、柳川宗平、西本祥仁、柴田護、鈴木則宏、西澤正豊、小野寺理
  • Organizer: 第52回 日本神経学会学術大会
  • Place of Presentation: 名古屋国際会議場 (愛知県)
• [Presentation] 孤発性ALSの運動神経細胞において特異的に出現する核内構造体の同定
  • Author(s): 西本祥仁
  • Organizer: 第54回 日本神経学会学術大会
  • Place of Presentation: 東京国際フォーラム
• [Presentation] ヒト胚性幹細胞由来運動神経細胞でのTDP-43発現による細胞毒性の検討
  • Author(s): 西本祥仁
  • Organizer: 第53回 日本神経学会学術大会
  • Place of Presentation: 東京国際フォーラム